The midline spinal cord hamartoma is a rare non-hamartomatous malformation that is often accompanied by a dermal sinus tract. Lipoma of the filum terminale is one of the subtypes of closed spinal dysraphism and it is characterized by lipomatous tissue that can inclose part or all of the filum terminale. A 5-year-old girl has soft tissue mass and hair growth that was present from birth in the posterior neck. In cervival MR examination, protrusion at the cervicomedullar junction and dermal sinus tract was observed. Also, in the lumber MR examination, lipoma of the filum terminale has shown. Midline spinal cord hamartoma is a extremely rare malformation and the lipoma of the filum terminale associated with this malformation have not been shown before.